Present Concepts of Steroid Therapy in Virilizing Adrenal Hyperplasia

From the Department of Pediatrics, The Johns Hopkins University School of Medicine and the Harriet Lane Home, The Johns Hopkins Hospital. Formerly Fellow of the National Foundation for Infantile Paralysis (Dr. Blizzard). Present address of Dr. Blizzard: Assistant Professor of Pediatrics and Assistant Professor of Medicine, Ohio State University Medical Center, Children's Hospital, Columbus, Ohio.

Since 1950, when cortisone was first used for the treatment of virilizing adrenal hyperplasia, the methods and results of therapy have been reported from time to time from this clinic 1-9 and by other workers,10-13 and the differential diagnosis also has been discussed.8,9 This paper summarizes briefly the present concepts concerning the pathogenesis and differential diagnosis of this disorder, and the results of treatment with cortisone and some of the newer closely related steroids.

As shown in Table 1, of 87 patients observed 68, or 78%, were female pseudohermaphrodites. It is probable that this does not represent the true sex distribution, as the diagnosis is made more readily in females, while males often die in infancy from salt loss before the true nature of the disorder is suspected. Thirty, or approximately one-third, of the patients had associated salt-losing symptomatology. Seventy-five patients have been treated, of whom only